Search

Bilateral adrenal neuroblastoma (NB) is rare and is mainly stage 4S. Its incidence, presenting features, management, and outcome have not been fully defined yet. We searched the Italian NB Registry (RINB) for stage 4S NB infants with bilateral adrenal primary tumor to compare them with stage 4S NB with unilateral tumor. Between 1979 and 2016, the RINB enrolled 3731 NB patients aged 0-18 years including 317 infants (8.5%) diagnosed with stage 4S NB. Eleven/317 (3.5%) had a bilateral adrenal primary tumor (Group 1) and 190/317 (59.9%) had a unilateral tumor (Group 2). Group 1 infants were significantly younger (51 vs. 89 days) but were comparable with Group 2 for any other presenting features. In the absence of specific protocols, upfront treatment was based on symptoms, size of adrenal tumors, and biology, and consisted of observation in 5 cases, radiotherapy in one, chemotherapy in 2, and surgery in 3. Five/11 developed progression and 2 of them, both with

Background Infants diagnosed with stage 4 s neuroblastoma commonly experience spontaneous disease regression, with few succumbing without response to therapy. We analyzed a large cohort of such infants enrolled in the Italian Neuroblastoma Registry to detect changes over time in presenting features, treatment and outcome. Methods Of 3355 subjects aged 0–18 years with previously untreated neuroblastoma diagnosed between 1979 and 2013, a total of 280 infants (8.3%) had stage 4 s characteristics, 268 of whom were eligible for analyses. Three treatment eras were identified on the basis of based diagnostic and chemotherapy adopted. Group 1 patients received upfront chemotherapy; Group 2 and 3 patients underwent observation in the absence of life-threatening symptoms (LTS), except for Group 3 patients with amplified MYCN gene, who received more aggressive therapy. Results The three groups were comparable, with few exceptions. Ten-year overall survival significantly increased from 76.9 to 89.7% and was worse for male gender, age 0–29 days and presence of selected LTS on diagnosis, elevated LDH, and abnormal biologic features. Infants who underwent primary resection ± chemotherapy did significantly better. On multivariate analysis, treatment eras and the association of hepatomegaly to dyspnea were independently associated with worse outcome. Conclusions Our data confirm that stage 4 s neuroblastoma is curable in nearly 90% of cases. Hepatomegaly associated to dyspnea was the most important independent risk factor. The cure rate could be further increased through timely identification of patients at risk who might benefit from surgical techniques, such as intra-arterial chemoembolization and/or liver transplantation, which must be carried out in institutions with specific expertise. Electronic supplementary material The online version of this article (10.1186/s13052-018-0599-1) contains supplementary material, which is available to authorized users.

This research explores how tangible interactive technology might offer opportunities for socialization and sensory regulation. We present a study carried out in an educational setting during leisure activities with a small group of children with autism who like music. We introduce Όλοι (pronounced Olly), a sonic textile Tangible User Interface (TUI) designed around the observations of five minimally verbal children with autism aged between 5-10 years. The TUI was tested for an average of 24 minutes once per week, over a period of five weeks in a specialized school based in North-East London, UK. We propose a methodological approach that embraces diversity and promotes designs that support repetitive movements and self-regulation to provide the children with a favorable environment and tools to socialize with peers. The findings show positive outcomes with regards to spontaneous social interactions between peers particularly when children interacted with or around Olly. These were observed in the form of eye-contact, turn-taking, sharing (of the space, the object and experience), and more complex social play dynamics like associative and cooperative play. We illustrate how the TUI was a positive stimulus of social behaviors and discuss design implications for novel technologies that aim to foster shared experiences between children with autism.

Playtime is an important activity for child development as it stimulates as well as predicts cognitive, motor, emotional and social skills. Children with autism may find it difficult to socialize, particularly initiating and maintaining human interactions. Consecutively, it is thought that playing with peers is often a challenge that many children avoid by simply playing in solitary mode. We present the design of Mazi, an e-textile sonic tangible user interface (TUI) designed with the aim of promoting basic social skills; stimulating spontaneous, independent and collaborative play; and providing sensory regulation opportunities. Mazi was tested in a Special Education Needs (SEN) School based in NorthEast London, with a group of five minimally verbal children with autism aged between 6 to 9. The results show great potentials for TUI implementation in educational settings as a way of promoting social skills through carefully designed playful and recreational activities.